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CME Sonography 96: Spleen Changes Abstract. The spleen is a somewhat neglected organ but there are some changes that must not be neglected. Splenomegaly, caused worldwide most commonly by malaria, can occur in other infections, in portal vein thrombosis or in portal hypertension in the context of liver cirrhosis. An accessory spleen is also often found. Sometimes after splenectomy, small remains are found which may later hypertrophy. Where focal changes are concerned, we differentiate between various forms of spleen cysts, lymphomas, metastases, and benign tumors.

CME Sonography 96/Answers: Spleen Changes Abstract. The spleen is a somewhat neglected organ but there are some changes that must not be neglected. Splenomegaly, caused worldwide most commonly by malaria, can occur in other infections, in portal vein thrombosis or in portal hypertension in the context of liver cirrhosis. An accessory spleen is also often found. Sometimes after splenectomy, small remains are found which may later hypertrophy. Where focal changes are concerned, we differentiate between various forms of spleen cysts, lymphomas, metastases, and benign tumors.

Medical History and Initial Presentation: A 35-year-old patient with a previous history of persistent episodic fever, sore throat, myalgia, and cephalgia presented for evaluation of pancytopenia. He had no recent travel history, except for a stay in Italy 1 year prior to admission and in Spain several years in the past., Diagnostic Workup: Laboratory evaluation confirmed pancytopenia, agranulocytosis, and elevated infection parameters without indicative serological results en par with lymphadenitis colli. Computed tomography scanning revealed cervical lymphadenopathy, hepatosplenomegaly, and colitis with occult perforation of the sigmoid colon. Bone marrow biopsy showed an infiltration of polyclonal plasma cells. Lymph node biopsy was compatible with necrotizing lymphadenitis., Diagnosis: Polymerase chain reaction analysis of a lymph node specimen confirmed the presence of Leishmania species, thereby enabling the diagnosis of visceral Leishmania., Therapy Course: Treatment with liposomal amphotericin B was initiated. Both fever and lymphadenopathy quickly resolved., Conclusion: VL is a clinically pleiotropic, severe disease with fatal outcome if left untreated. It often presents with distinct similarities to hematologic malignancies. Exacerbation can occasionally occur as fulminant macrophage activation syndrome. Disease incidence is globally increasing and has not peaked as yet. A complex interplay between pathogen and the immune system is the key pathophysiological mechanism.

Unlabelled: In textbooks about abdominal sonography, the parenchyma of the spleen is described as homogeneous. However, using high-frequency probes, sonography of the spleen in children often reveals a micronodular pattern., Aim: To investigate the prevalence of a micronodular pattern in the pediatric spleen and to evaluate possible correlations to clinical parameters., Methods: In 106 non-selected children (54 males, 52 females, mean age 7 years 10 months), the spleen was examined with a 17 MHz probe. Simultaneously the size of the spleen was measured, and other parameters such as leukocyte and thrombocyte counts were recorded. The pattern of the spleen was estimated using a four-step scale. 0: homogeneous tissue without micronodular components, 1: faint micronodular pattern/barely perceptible, 2 and 3: micronodular pattern clearly visible of moderate (2) or strong intensity (3)., Results: In 33 children a homogenous pattern was found (31.1 %) and in 48 children (45.3 %) a micronodular pattern of the spleen was clearly visible. A statistical correlation to spleen size or leukocyte or thrombocyte count or CRP could not be proven. 52 % of children with an enlarged spleen had a micronodular pattern, as well as 66.7 % of children with leukocytosis, 44.7 % of children with increased CRP, and 54.5 % of children with thrombocytosis. However, there was a strong statistical correlation to the age of the children with a maximum at the age of 1 to 5 years., Conclusion: A micronodular pattern of the spleen is frequent in children, even without clinical signs of infection. It is important to be aware of this finding and not to confuse it with pathological changes., (© Georg Thieme Verlag KG Stuttgart · New York.)

We proceeded to an extensive etiologic search in a young women with a hepatosplenomegaly and a chronic persistent fever. We discuss the differential diagnosis of this situation with a final diagnosis of sarcoidosis.

History: A 27-year-old woman from Cameroon was admitted because of arthralgia, myalgia and severe thrombocytopenia (20,000/ micro l). She had been suffering from weakness, recurrent febrile episodes, generalized lymphadenopathy and pancytopenia for 2 years. Having a typical autoantibody constellation and fulfilling four (pleurisy, autoimmune-hemolytic anemia, antinuclear antibodies (ANA), anti-Sm antibodies) of the American College of Rheumatology (ACR) classification criteria, systemic lupus erythematosus (SLE) had been diagnosed at another hospital. Treatment with corticosteroids and azathioprine did not improve her condition., Investigations: Abnormal laboratory findings were pancytopenia, elevated markers of inflammation and extreme hypergammaglobulinemia (70 %) with polyclonal IgM (73 g/l). Antinuclear antibodies (ANA), anti-Sm-, anti-Scl 70-, anti-U1-RNP-, anti-histo-, anti-leukocyte- and IgM anticardiolipin antibodies were detected. A bone marrow biopsy showed polyclonal B-cell and plasma cell infiltrates. Examination of peripheral blood smears disclosed trypanosoma brucei infection., Diagnosis, Treatment and Course: After the diagnosis of stage 2 West African trypanosomiasis (sleeping sickness) specific treatment was initiated leading to subsequent remission of the disease., Conclusion: This case report underlines the importance of a thorough differential diagnosis in cases of suspected autoimmune disease. Induction of autoantibodies during infectious diseases may be misleading. The use of the ACR criteria for SLE must be restricted to the classification of proven connective tissue diseases.

History and Admission Findings: A 17-year-old girl with a history of a polyarthritis of unknown etiology was admitted because of acute fever and general weakness. There were palpable cervical lymph nodes and her body temperature was 39.5 degrees C., Investigations: GOT was raised to 282 U/1, GPT to 266 U/l lactate dehydrogenase to 1275 U/I and bilirubin to 0.6 mg/dl. The Quick value was 67%, albumin 28 mg/dl. White cell count was decreased to 1700/microl, with 43% granulocytes, 39% lymphocytes, 17% monocytes. Platelet count was 64,000/microl. Ultrasound revealed splenomegaly. Ferritin was markedly raised to 11,860 ng/ml (normal up to 150 ng/ml). An epstein-barr-virus infection was found., Therapy and Clinical Course: Suspecting a reactive hemophagocytosis syndrome, she was treated with prednisolone (2 mg/kg). The diagnosis was confirmed by a bone marrow aspirate. The patient's condition and laboratory values improved rapidly., Conclusion: Markedly increased ferritin levels in a clinically septic patient with an underlying rheumatic disease indicates a hemophagocytotic syndrome. High dosage steroid should be started before there is biopsy confirmation of the disease.

Purpose: The weight of the spleen can be easily calculated from sonographic measurements. This study investigates the correlations between the weight of the spleen determined sonographically and the weight measured at autopsy or after splenectomy., Materials and Methods: The splenic weight at autopsy or after splenectomy was correlated with the calculated sonographic splenic weight in 55 patients who had a sonographic examination within the preceding three weeks., Results: A significant correlation (r = 0.978) was found between the sonographically calculated splenic weight (460.82 grams) and the measured weight of the removed spleen (average weight 463.18 grams). Spleens weighing more than 1 kg showed a slightly weaker correlation., Conclusion: This study proves that the simple formula of length x transverse diameter x vertical diameter x 0.6 provides a reliable calculation of the weight of the spleen.

We describe a case of Niemann-Pick disease type B. A 13-year-old female adolescent of Turkish origin suffered from abdominal pain for several months, finally leading to hospitalisation. The investigations revealed splenomegaly and interstitial pneumopathy. The bone marrow contained giant foam cells typical of Niemann-Pick disease. Enzymatic analysis of a fibroblast culture confirmed the diagnosis of Niemann-Pick disease type B, with reduced activity of acid sphingomyelinase. Niemann-Pick disease is an inherited autosomal recessive lysosomal storage disorder of sphingolipids, resulting in an accumulation of sphingomyelin in the cells of the reticulo-histiocytic system due to an enzymatic defect. In Niemann-Pick disease type B the spleen and lung are the main organs affected. At present no treatment exists for this disorder.

Osteomyelofibrosis is a myeloproliferative disorder in which fibrosis and sclerosis finally lead to bone marrow obliteration. Liver and spleen compensate for bone marrow loss with extramedullary hematopoiesis. In some patients the resulting splenomegaly causes severe symptoms such as local compression, thrombocytopenia and hemolytic anemia. In such patients, splenectomy is the only promising treatment, although it represents a significant risk.

Aim of Study: The haemodynamic role of the spleen in portal hypertension remains unclear. Duplex sonography was undertaken prospectively to discover the relationship of splenomegaly and splenic vein flow to type and severity of liver cirrhosis and portal vein flow, as well as to the degree of oesophageal varices., Patients and Methods: 89 patients (54 men, 35 women; mean age 52 [26-81] years), diagnosed in the second half of 1993 as having liver cirrhosis were consecutively included in the study. In 40 patients the cirrhosis was in stage A (according to Child-Pugh classification), in 31 in stage B and in 18 in stage C., Results: Portal vein flow fell significantly with increasing degree of cirrhosis (stage A: 8.29 cm/s; stage B 7.03 cm/s; stage C 5.26 cm/s; P < 0.05). Splenic vein flow differed significantly only between stages B and C (stage A: 9.22 cm/s; stage B: 9.46 cm/s; stage C: 7.87 cm/s; B vs C, P < 0.05). There was no correlation between portal vein flow, splenic vein flow, degree of splenomegaly and extent of oesophageal varices., Conclusion: The results may be explained by the presence of collateral circulations and by the differing pathophysiological part played by the spleen in liver cirrhosis of different aetiologies.

The POEMS-syndrome is a very rare disorder, of which not much more than 100 cases are known worldwide, mostly in Japan. The following case report depicts the typical radiological changes to the skeletal system and inner organs.

Splenomegaly, ascites, and anatomy of intra- and extrahepatic portal vessels can reliably be detected by ultrasound in case of portal hypertension. The increased diameter of the portal vein and its roots is a not sufficient sensitive and specific finding in portal hypertension. However a marked variation of diameter at the superior mesenteric vein during in- and expiration is nearly exclusive. With the help of colour flow imaging or duplex sonography additional finding of blood flow in the portal system can be detected noninvasively and continuously. Quantitative blood-flow measurement in routine examinations is unnecessary and reserved to special questions. Reverse flow or significantly decreased blood flow velocity or the detection of portocaval collaterals are reliable findings in portal hypertension. In addition thrombosis of portal vessels and its hemodynamic consequences can be seen. Because underlying diseases e.g. liver cirrhosis or tumours are diagnosed in the same procedure ultrasound techniques are used in first line when portal hypertension is suspected. The findings are complementary to endoscopy of upper g.i.tract and lead on the one hand to a well-aimed use of CT scanning or x-ray splenoportography, and--on the other hand--make them dispensable in a low of cases.

The purpose of this study was to correlate CT-assessed spleen indices (length x width x depth) and their changes under therapy or in the follow-up, with the clinical course and morphological changes. In 66 patients with 200 CT's of the abdomen the spleen index was calculated and 134 spleen index differences (delta SI) were correlated with the clinical outcome. The normal group showed a mean of delta SI of -8 = 112, equivalent boundaries of -120 and 104. delta SI of progressive lymphomas (n = 32) was 313 (p < 0.01). In 10/14 patients of this group, morphological signs of infiltration were found. In regressive lymphomas (n = 63) we found delta SI = -211 (p < 0.001). 23/29 with delta SI < -120 showed signs of spleen involvement in the preliminary examination. Since there are only slight intraindividual changes of CT-assessed spleen indices and a good correlation of spleen index differences with the clinical outcome. the spleen index is a useful instrument in the follow-up of malignant lymphomas.

Splenomegaly is a common finding in patients with portal hypertension. In the present study the relation between spleen size and blood flow in the splenic and portal vein was evaluated in 33 patients with alcoholic liver cirrhosis and portal hypertension using pulsed Doppler sonography (Ultramark 9, ATL, Solingen, FRG). There was a significant positive correlation between hilar spleen diameter (HD) and splenic vein diameter (r = .73, p less than .001) as expected as the consequence of portal hypertension. However, a positive correlation between HD and splenic vein flow (SBF) was found (r = .67, p less than .001). Furthermore, there was no negative correlation between HD and flow velocity in the splenic vein (r = .01, n.s.). Portal blood flow (830 +/- 360 ml/min) was fairly constant in spite of considerable variations in SBF (range: 120 to 1200 ml/min). The data of the present study indicate that splenomegaly in patients with liver cirrhosis and portal hypertension is not simply the consequence of portal congestion resulting in decreased SBF. Rather, increased SBF serves to maintain portal blood flow and thereby contributes to portal hypertension. In few patients (15%) SBF increased to more than 11/min may be an important factor for the severity of portal hypertension. Surgical shunt treatment should be adjusted in these patients.

Splenic infarcts are represented by wedge-shaped, oval or linear areas. Haemorrhagic infarcts are characterised by being hyperdense. Disseminated infarction occurs predominantly in myeloproliferative diseases. During the early stages, the infarct appears as an ill-defined hypodense defect, with non-homogeneous contrast enhancement. During the acute and sub-acute stage, the density of the infarct is low and there is no contrast enhancement. During the chronic stage, its density increases and there is slight contrast enhancement. Complications following splenic infarcts, such as abscesses, bleeding and rupture can be demonstrated by CT with great accuracy. Problems in differential diagnosis may occur if there are atypical manifestations of the infarct, with respect to abscess or leukaemic infiltrations.

Analysis of criteria used in the evaluation of left upper abdominal masses seen on CT. A very critical approach is recommended concerning the diagnostic reliability of density measurements and contrast-enhancement. The configuration of the mass and its relationship to the neighborhood seem to be more important aids for a reasonable differential diagnosis.

The combined use of selective angiography, scintigraphy and ultra-sound is not reliable enough for the certain exclusion of visceral involvement in Hodgkin's disease. Since this is of importance in the planning of treatment, it confirms the recommendation made by Wagenknechts, Glatsteins and others that a diagnostic splenectomy be carried out in order to determine the presence of visceral involvement.

The evaluation of spleen length from routine x-ray films of the left epigastrium of 97 healthy patients suggested a close correlation to the height of the 12th thoracic and the first three lumbar vertebral bodies. The normal ratio of spleen length to the vertebral height Th 12- L 3 is 0,82 +/- 0,09, two standard deviations are represented by ratios from 0,64 to 1,0. Spleen length over vertebral height Th 12 -L 3 indicates pathologic enlargment of the spleen. 54 patients with diseases which may cause splenomegaly showed spleen lengths mostly over the heights Th 12 - L 3.

Report of a case of unusual renal displacement due to massive splenomegaly. The left kidney was displaced upwards by massive enlargement of the spleen. The own findings of ultrasonography, abdominal computed tomography and intravenous urogram are described and compared with the findings in the literature.